Oral Pathology Chapter 16 Flashcards
What is the name of the pathology that is a group of inherited conditions in which two or more ectodermal anatomic structures fail to develop?
Ectodermal dysplasia
What is the best known type of ectodermal dysplasia?
Hypohidrotic Ectodermal Dysplasia
What pathology is an autosomal dominant disease that results from defects in keratins and appears as thickened, symmetrical, white, corrugated, diffuse plaques on the bilateral buccal mucosa?
White Sponge Nevus
What is the scientific term that describes the idea of a genetically determined skin disorder?
Genodermatosis
What pathology is recognized by freckle-like lesions on the hands, perioral skin, and oral mucosa?
Peutz-Jeghers Syndrome
What side effect does Peutz-Jeghers Syndrome have?
Intestinal Polyposis
What is the significance for patients that develop Peutz-Jeghers Syndrome?
They have a predisposition to develop cancer
What type of growth are the intestinal polyps in Peutz-Jeghers Syndrome?
Hamartomatous
What digestional problem do patients with Peutz-Jeghers Syndrome often have?
Intussusception (proximal bowel becomes telescoped into distal portion)
How much more likely are Peutz-Jeghers syndrome patients to develop a malignancy later in life than an ordinary person?
18 X more likely
What other problem develops in the digestion tract for Peutz-Jeghers Syndrome patients?
GI adenocarcinomas develop (note that they do NOT develop from polyps)
What pathology is associated with CNS manifestions, mental retardation, seizures, potato-like growths, angiofibromas of the skin, ungual fibromas and ash-life/shagreen patches on the skin?
Tuberous Sclerosis
What rare side effects can be associated with Tuberous Sclerosis?
Cardiac Rhabdomyoma
What effect does Tuberous Sclerosis have on the kidneys?
Angiomyolipomas form there
What are the oral manifestations of Tuberous Sclerosis (2)?
- Developmental enamel pitting on facials of anterior permanent teeth and 2. Multiple Fibrous Papules
What are the odds of a patient with Tuberous Sclerosis developing tubers, seizures and mental retardation?
85%, 75% and 40%
What disease is related to autoantibodies against the desmosomes?
Pemphigus Vulgaris
What skin lesions are present in Pemphigus Vulgaris (2)?
- Flaccid vesicles and 2. bullae
What is the name of the sign that signifies the induction of bulla on normal-appearing skin?
Nikolsky sign
What is the term given for the “falling apart” of epithelium in Pemphigus Vulgaris?
Acantholysis
What is the name of free-floating cells found in Pemphigus Vulgaris and some other pathologies (Herpes, etc.)?
Tzanck Cells
What is the name of the familial disease that passes Pemphigus Vulgaris around in the same family?
Hailey-Hailey disease
What disease causes problems in hemidesmosomal binding and creates vesicles/bullae?
Mucous Membrane Pemphigoid
What intraoral lesion is almost pathognomonic for Mucous Membrane Pemphigoid?
Intraoral blood blister
What is the other term that is used for Mucous membrane pemphigoid?
Cicatricial Pemphigus
What is the most significant complication of Mucous membrane pemphigoid?
Ocular Involvement (25%)
Scarring in Mucous Membrane Pemphigoid causes what to occur in the eyelids?
Eyelids turn inward on themselves (Entropion)
What two proteins are found along the basement membrane in Mucous Membrane Pemphigoid?
C3 and IgG
What disease is a blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis?
Erythema Multiforme
What can be said of 50% of Erythema Multiforme cases?
Clinicians can identify a preceding infection or exposure to a new medication
What two infections have the greatest chance of developing into Erythema Multiforme?
- HSV and 2. Mycoplasma pneumoniae
What percentage of Erythema Multiforme patients have experienced a HSV infection previously?
50%
What three gradients of Erythema Multiforme have been classified?
- Erythema multiforme minor, 2. Erythema Multiforme major and 3. Toxic epidermal necrolysis
What is the more common name used for Erythema Multiforme Major?
Stevens-Johnson Syndrome